INTRODUCTION:Malignant chondroid syringoma(MCS) is exceedingly rare. MCS metastasis occurs by hematogenous spread and lymphatic. There has been only one case of biopsy proven lung metastasis in world literature. We report a case of pulmonary metastasis that occurred 22 years after the primary diagnosis.
CASE PRESENTATION:45-year-old male presented with complains of left sided chest pain for the past 2 ½ years. The pain had gotten worse over the last 6 months with worsening pain on deep inspiration and with cough. The pain radiated to the back with some dyspnea and difficulty sleeping on the left side due to pain. Patient also complained of mild intermittent hemoptysis for over a year. Chest X-ray 6 months ago had shown a left lung mass. Patient had a 20 pack year smoking history and worked as an auto mechanic. Past medical history was significant for a history of chondrosarcoma of the right soleus muscle diagnosed in 1984, treated surgically. He had a recurrence 4 years later behind his right knee, underwent excision followed by chemotherapy and radiation therapy. Since then, he was told to be cured and did not have any follow up. A positron emission tomography/CAT scan showed a pleural based mass at the posterior aspect of the left lung measuring 5.3 × 4.4 cm demonstrating hypermetabolic activity with standardized uptake values(SUV) values measuring upto 6.8 consistent with malignancy. Chest wall invasion and osseous rib destruction was also seen. The fine needle biopsy showed it to be malignant chondroid syringoma. Patient underwent left thoracotomy and left lower lobe resection of the tumor and positive margins were noted. On review of pathology slides from 1984 and 1988 showed the tumor to be similar in appearance. The patient received radiation therapy due to the large tumor size and positive margins.
DISCUSSIONS:Malignant chondroid syringoma (MCS), a very rare malignancy is a mixed tumor of the skin, originating in sweat glands. Most reported cases of chondroid syringoma have been benign and were first described by Hirsch and Helwig in 1961. Presence of the “sweat gland elements” and the prominent component of cartilage like material in most tumors, has lead to the name “chondroid syringoma”. MCS with metastasis is a rare malignancy with a total of 30 case reported. MCS occurs mostly in women and is typically seen in the extremities and torso. Our patient presented with long standing left sided pain with recent worsening. The latency between the initial tumor and the metastasis is typical of a slow growing tumor like MCS. Case reports of metastasis presenting more than 20 years after the initial tumor have been reported. In contrast to their benign counterparts, MCS occurred mainly in the extremities (18 of 30 cases, 60%) (1). The median age was 49 years, with a range from 13 to 83, with 19 females and 11 males. MCS metastasizes by lymphatic and hematogenous spread. Local spread and bony metastasis are less common. Hence, the preoperative evaluation should consist of computed tomography of the neck, chest, abdomen, and pelvis. Adequate wide surgical excision is the cornerstone of treatment for MCS.
CONCLUSION:Metastasis to the lung from MCS is very rare. To our knowledge, this is the second biopsy proven case of MCS with metastasis limited to lung. The disorder is difficult to diagnose considering the rarity and the slow growth. The case emphasizes the importance of a detailed history, complete examination and necessity of obtaining previous biopsy specimens especially where an unusual tumor is suspected.
DISCLOSURE:Aamer Rahman, No Financial Disclosure Information; No Product/Research Disclosure Information